Fig. 1. Simplified metabolic pathways associated with isocitrate dehydrogenase (IDH) and 2-hydroxyglutarate (2HG).

Mutations of IDH1 and IDH2 result in overproduction of 2HG. 1H-MRS-visible metabolites including 2HG are marked in bold. Acetyl-CoA = acetyl coenzyme A, Gln = glutamine, Glu = glutamate, GSH = glutathione, GSSG = glutathione disulfide, H₂O = water, H₂O₂ = hydrogen peroxide, IDH-MT = mutant isocitrate dehydrogenase, NAD = nicotinamide adenine dinucleotide, NADH = nicotinamide adenine dinucleotide hydrate, NADP = nicotinamide adenine dinucleotide phosphate, NADPH = nicotinamide adenine dinucleotide phosphate hydrate, α-KG = alpha-ketoglutarate